Wednesday, July 26, 2017

day +204 post hsct update

Remember when I said, "lets hope it isn't gastroparesis?" Well, turns out, it's gastropareis. Bummer. Unfortunately, the digestive issues I was hoping would chalk up to some sort of virus was definitely delayed gastric emptying, aka gastroparesis. This is often a comorbidity of scleroderma, and is the next logical sign of progression after esophageal paralysis. This has lead my team to believe that my systemic sclerosis is once again active, which would garner a diagnosis of relapse post HSCT. However, I am still holding out hope this is just a bump in recovery since I am so young post HSCT. While my docs are pretty much convinced my transplant has worn off, I am optimistic that with the proper immune suppression, we can hold off, or at least slow any further damage that will accrue.

July 

  • My gastric emptying study shows moderately delayed emptying. The nerve that tells the soft muscles to move, called peristalsis, is damaged - so it's not sending the correct signal to move food through to the intestines from my stomach. This causes my stomach to hold onto food longer than it should, equaling serve cramping, distention, bloating, vomiting, and nausea. Basically, the food stays undigested in my body until I eject it. Gross. Unfortunately, this requires the use of a feeding tube that bypasses the stomach so I can get proper nutrition. 
  • I saw my GI physician post GES, who wasn't fabulously optimistic I would get to continue my life without a permanent feeding tube. She schedule a GJ feeding tube surgery for about six weeks out, that way we have some time to trial a few medications. She prescribed a med called Domperidone, which essentially moves the stomach muscles for you; it also blocks the receptor in the brain that controls nausea. The medication isn't FDA approved even though thousands of patients use it for gastroparesis. So while I wait for it to ship from Canada, I am trying out Erythromycin - an antibiotic used to speed up gastric emptying. I take it around the clock, but especially before I try to eat anything. Unfortunately, these drugs are subject to the consumer, which means they may or may not work. So far, I have seen a little improvement from the Erythromycin, but not enough to eat an entire meal. Just a light snack, or drink - both of which have to follow the strict gastoparesis diet of low fat/fiber. I have experienced much less nauseas since starting the meds at least, so thats a win! Unfortunately, that means I am stuck with my NJ tube until surgery placement, or until/if the domperidone works. Urg. Chances are I will need the tube, and the domperidone, but in a perfect world, I would be able to eat and not need the tube! 
  • We also scheduled an appointment with my Pulmonologist, Rheumatologist, and Autonomic Specialist. Since my DLCO decreased by 22%, and the swallow study showed I am not aspirating, we are now worried about systemic sclerosis lung progression. I will be repeating a PFT here with my Pulm, and following up to diagnosis whatever this issue may be. My Rheum will probably throw me back on some immunosuppressive treatments to hold off this damn disease until we have a better option, and my Autonomic Specialist can hopefully get my increased Dysautonomia symptoms under control. I'm exhausted mentally, but physically feeling okay!
  • I spoke at the National Scleroderma Conference in Phoenix last weekend. I always enjoy connecting with other patients, and hearing about all the new medical advances! 

I'm going to be taking a short social media break. I just feel I need to focus on other things right now. A little cleanse/detox from the turbulent online world is good for the soul sometimes. It kind of breaks my heart, since I absolutely LOVE connecting with other patients and my readers - but please understand, this is for my mental health. Transitioning back to the world of chronic illness after have such an incredible, trouble free 6 months has been mentally, and physically tasking - but mostly mentally. I will not be answering messages, or comments until further notice. I am so grateful for the love and support I constantly receive, but right now I just need to focus on what's best for my overall health. I may occasional post a quick update, but won't be divulging into replies. I promise I will be back soon! Thank you for your understanding at this time. 


xoxo Chanel

Wednesday, July 12, 2017

Day +190 post hsct update

The last three weeks have been a bit of an emotional roller coaster. I have been feeling so out of sorts - kind of like I am a puzzle piece that just can't find its place in the picture. I am happy with being a puzzle piece, but I desperately feel left out and confused. Does that make sense? lol Any-who, my body has been an absolute piece of work since mid Jun, so I actually have quite a bit to update. The last week of June I headed to Chicago to see my HSCT team for a six month follow up, and it was a raging disaster. Yikes.

June

  • The last week of June I trekked out to Chicago to see my transplant team for a six month post HSCT follow up. Remember, I signed my life away for 5 years to participate in this study, which means I have to return to Chicago once a year to update the doctor on my progress for research purposes. I basically got one piece of bad news after another the entire trip. I saw the researching physician, the pulmonologist, and the gastroenterologist. I also had blood work, a PFT, an EKG, and a last minute angio-CT preformed. My blood work showed my autoimmune markers didn't change a bit (ANA still tittering at 1:1280), my DLCO actually dropped 22%, my skin and raynauds somehow worsened, and my GI tract decided to crap out on the trip which resulted in many sleepless nights vomiting undigested food. Now to be fair, everything was going swimmingly for the first 5 months, but for some reason in June all hell broke loose. I started having a lot of nausea/bloating while eating, uncontrolled diarhea, and on the trip started throwing up about 3-4 hours after meals. My fingers noticeably tightened, and I just couldn't get my raynauds under control. To top it all off I seem to be aspirating quite severely upon ingesting anything, accompanied by chest tightening. All the physicians agreed I was having a relapse - this is fancy terms for 'my disease was once again progressing', which isn't suppose to happen post HSCT. Basically, the whole trip consisted of everyone telling me how "unfortunate it was that the effects of my transplant only lasted 6 months." *Insert tears and eyeroll here* While I knew the transplant wasn't permanent, I figured I would get more than a simple six months of relief! SIGH. I in no way regret the transplant, and still believe it was successful... just not as lasting as we thought. Many things did improved tremendously, like my esophagus! I was thrilled to have the six months of decreased symptoms I did - no matter how short lived! I have spoken to a few patients that said this happened to them as well, so at least I know I am not alone. My hope is that even though I am progressing again, it will move slower than my original timeline, which lead to organ failure in a mere two years. 

July

  • Due to the severity of the digestive issues my transplant team sent me back with loads of test to preform here in Seattle. The tests due to be completed are: an aspiration assessment, a gastric emptying study to asses stomach function, and a small bowel follow through to asses intestinal function. The tests will help us better understand what organs are malfunctioning, and what type of tube I will need moving forward - or if possibly, all these digestive issues are of a more transient nature due to a virus, or possibly even Graft Vs Host. (Fingers crossed for transient!) In the interim, my GI physician placed a NJ (Naso-Jejunal) tube. This is a feeding tube which is inserted through the nose, fished through the esophagus, past the stomach, and into the intestines. Due to my suspected aspiration she wanted a tube that bypassed the possibility of aspiration, while still providing nutrition until we have the situation assessed. Honestly, my digestion by that point had gotten so bad I welcomed the horrible little device. I was so light headed from malnutrition I was unable to drive myself around upon retuning from Chicago! It has given me the much needed nutrition I had been deprived of the previous two weeks. 
  • Monday I had the aspiration assessment preformed in fluoroscopy. The test was done by a speech therapist who uses an x-ray machine to watch different consistencies of barium pass through the oral, pharyngeal, and esophageal phase of swallowing. To my (and my docs) surprise, she found I did not aspirate - None of the fluid ended up in my lungs! Actually, compared to the exact study I had done about two years ago my esophageal muscles had improved greatly! Liquid was able to pass through the esophagus - While I still couldn't pass solids such as pudding, being able to pass liquid was a huge improvement post transplant! What she did find regarding my coughing fits and lighted headedness while eating however, is baffling! Luckily, during the test I had what we all assumed was aspiration, included: a deep whooping cough, facial flushing, and a tightness of chest accompanied by feeling faint. The speech therapist watched on the monitor and saw what actually was happening was called a Laryngospasm. This is categorized as severe vocal chord dysfunction, where a spasm of the larynx causes your airway to close! (Yes it's as unpleasant as it sounds...) For reference, the larynx (aka voice box) protects the trachea from aspiration. In short, my vagus nerve is malfunctioning, telling my larynx that I am aspirating, causing it to close - even though I am not! Literally baffling. The vagus nerve is part of the autonomic nervous system which regulates unconscious actions such as digestion, heart rate, breathing, etc. The speech pathologist explained to me that I have Vagus Neuropathy, meaning I have damage to that system, causing inappropriate reactions. Because this nerve is responsible for digestion, when I eat, it triggers an inappropriate reaction in the larynx - just for fun. SERIOUSLY?! Can my body just chill for like two seconds?! She actually believed this may be what is causing my other current digestive issues as well! Good news is, the Vagus Neuropthay might be manageable! If thats the case, I might be able to get rid of this darn nose hose and not need another feeding tube surgically placed!
Tomorrow I have my gastric emptying study to asses my stomach motility. This is the gold standard test to diagnosis gastroparesis, aka stomach paralysis. This is often a comorbidity of scleroderma, and besides esophageal paralysis, is the number one reason a patient requires a feeding tube. I already feel my motility has improved a bit since we ordered the test a week ago, so here is to hoping all the vomiting and nausea was due to some crazy virus and not gastroparesis! However, gastroparesis can be a "flare" illness, meaning the paralysis can come and go - when caused by, you guessed it, a faulty vagus nerve! Guess I'll find out tomorrow! For more detailed and up to date information don't forget to check out my Facebook page!